Rapidly involuting congenital hemangioma associated with Kasabach-Merritt Syndrome

Ballona, Rosalía; Zevallos, Jean; Núñez, Jeanette

doi:10.29328/journal.japch.1001032

Cited by 1 publication

(2 citation statements)

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“… 16,19‐22 In the presence of cardiac failure, the mortality rate in such cases has been reported as high as 30% 18 . When patients do require treatment, case reports have focused on embolization, excision, and ligation 6,13,17,18 . However, cardiac complications may preclude the anesthesia necessary for intervention and also drastically increase the risk of any procedural approach.…”

Section: Discussionmentioning

confidence: 99%

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Catastrophic congenital hemangioma with severe coagulopathy leading to fatal cardiac failure: Case report and review

Cohen‐Cutler

Szymanski

Bockoven

et al. 2021

Pediatric Dermatology

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Congenital hemangiomas (CH) are rare vascular tumors of the neonate, with an estimated incidence of less than 0.5%. 1 CH are present at birth and may involute to varying degrees over the first 2-12 months of age. 2 Based on the course and degree of involution, CH are subdivided into rapidly, partially, and non-involuting subtypes. [3][4][5] Complications of CH are rare and primarily related to high-flow vasculature that may initially be present before the involution phase begins. The most common sequela of this includes ulceration and bleeding, particularly in the peripartum period. 6,7 High-output congestive heart failure can rarely result from intralesional shunting in larger CH, particularly those within visceral organs. Increased blood flow can also lead to a mild coagulopathy, evidenced by hypofibrinogenemia and thrombocytopenia. 2,[8][9][10][11] These findings are usually transient and less severe than Kasabach-Merritt phenomenon (KMP), which occurs most commonly in patients with kaposiform hemangioendotheliomas (KHE) and less so with tufted angiomas (TA). 12 Typically, CH can be managed supportively and expectantly.However, in cases requiring intervention, a lack of evidence in favor of medical therapies was found. 2 Corticosteroids, propranolol, and vincristine have been used with minimal success, and only embolization and surgical resection have been shown to be definitive. 9,13,14 We present a rare, and sadly lethal, case of a neonate with a large CH and severe, overwhelming coagulopathy and multiorgan failure.Treatment options, which were carefully weighed for our patient, are

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Section: Discussionmentioning

confidence: 99%

“…However, in cases requiring intervention, a lack of evidence in favor of medical therapies was found 2 . Corticosteroids, propranolol, and vincristine have been used with minimal success, and only embolization and surgical resection have been shown to be definitive 9,13,14 …”

Section: Introductionmentioning

confidence: 99%